FKBP8 Enhances Protein Stability of the CLC-1 Chloride Channel at the Plasma Membrane

Mutations in the skeletal muscle-specific CLC-1 chloride channel are associated with the human hereditary disease myotonia congenita. The molecular pathophysiology underlying some of the disease-causing mutations can be ascribed to defective human CLC-1 protein biosynthesis. CLC-1 protein folding is...

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Veröffentlicht in:	International journal of molecular sciences 2018-11, Vol.19 (12), p.3783
Hauptverfasser:	Peng, Yi-Jheng, Lee, Yi-Ching, Fu, Ssu-Ju, Chien, Yun-Chia, Liao, Yi-Fan, Chen, Tsung-Yu, Jeng, Chung-Jiuan, Tang, Chih-Yung
Format:	Artikel
Sprache:	eng
Schlagworte:	Biosynthesis Biotinylation Cell surface Chaperones Chlorides Endoplasmic reticulum Fractionation Hereditary diseases Immunoblotting Localization Membrane proteins Membranes Muscles Mutation Myotonia Plasma Protein biosynthesis Protein folding Proteins Quality control Sedimentation & deposition Skeletal muscle Sucrose Tacrolimus Tacrolimus-binding protein
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